1. Long-term preclinical magnetic resonance imaging alterations in sporadic Creutzfeldt-Jakob disease
- Author
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Sergio Ferrari, Michele Fiorini, Annachiara Cagnin, Salvatore Monaco, Giulia Camporese, Luca Santelli, Matilde Bongianni, Gianluigi Zanusso, and Renzo Manara
- Subjects
0301 basic medicine ,Gait instability ,Pathology ,medicine.medical_specialty ,medicine.diagnostic_test ,business.industry ,Magnetic resonance imaging ,Sporadic Creutzfeldt-Jakob disease ,Asymptomatic ,03 medical and health sciences ,Occipital Cortices ,030104 developmental biology ,0302 clinical medicine ,Neurology ,medicine ,Neurology (clinical) ,Gene sequence ,Cognitive decline ,Prion protein ,medicine.symptom ,business ,030217 neurology & neurosurgery - Abstract
An asymptomatic 74-year-old woman, on follow-up for a carotid body tumor, showed magnetic resonance imaging (MRI) focal restricted diffusion confined to the left temporal and occipital cortices. Thirteen months later, diffusion-weighted images revealed a bilateral cortical ribbon sign involving all lobes. After 1 month, the patient developed gait instability and cognitive decline rapidly evolving to severe dementia and death within 3 months. Prion protein gene sequence, molecular, and neuropathological studies confirmed the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) MM1 subtype. Here we show the kinetics of MRI changes and prion spreading in preclinical sCJD MM1. Ann Neurol 2016;80:629-632.
- Published
- 2016